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| LETTER TO EDITOR |
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| Year : 2013 | Volume
: 61
| Issue : 6 | Page : 680-683 |
Single, small, spontaneous, accessory, closed type, frontal sinus pericranii in a child: Favorable outcome with surgical excision
Amol Raheja, Guru Dutta Satyarthee, Bhawani Shankar Sharma
Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, New Delhi, India
| Date of Submission | 29-Aug-2013 |
| Date of Decision | 04-Nov-2013 |
| Date of Acceptance | 04-Dec-2013 |
| Date of Web Publication | 20-Jan-2014 |
Correspondence Address:
Guru Dutta Satyarthee
Department of Neurosurgery and Gamma Knife, All India Institute of Medical Sciences, New Delhi
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.125380
How to cite this article:
Raheja A, Satyarthee GD, Sharma BS. Single, small, spontaneous, accessory, closed type, frontal sinus pericranii in a child: Favorable outcome with surgical excision. Neurol India 2013;61:680-3 |
How to cite this URL:
Raheja A, Satyarthee GD, Sharma BS. Single, small, spontaneous, accessory, closed type, frontal sinus pericranii in a child: Favorable outcome with surgical excision. Neurol India [serial online] 2013 [cited 2023 Jun 2];61:680-3. Available from: https://www.neurologyindia.com/text.asp?2013/61/6/680/125380 |
Sir,
Sinus pericranii is a vascular anomaly presenting mostly in childhood It is a well-circumscribed, soft compressible mass consisting of venous malformation with abnormal communication between intracranial and extra-cranial venous channels though a precise calvarial defect, usually located in midline over frontal, parietal or occipital bone along sagittal sinus and very rarely reported in temporal bone or along transverse sinus. [1],[2],[3] We describe one such a rare case.
A 9-year-old boy presented with gradually progressive swelling over forehead 6 cm above glabella with no history of trauma. The swelling was non-pulsatile 5 cm × 4 cm × 3 cm soft, fluctuant, compressible and completely reducible on standing erect [Figure 1]a. With cough, on lying down and bending forward the lesion would become tense. There was no bruit.
Non-contrast computed tomography scan of head revealed a large homogenous isodense soft-tissue collection (5 cm × 4 cm × 3 cm) in the right frontal region [Figure 1]b. Bone window revealed defect in diploe [Figure 1]b. Contrast magnetic resonance imaging brain [Figure 1]c and d and percutaneous direct puncture venogram [Figure 1]e confirmed the diagnosis of closed type (non-drainer) sinus pericranii. Patient was electively operated after informed consent from parents. Patient was taken up for surgery and intra-operatively there were trans-diploic dilated venous channels with communication between cortical draining veins into superior sagittal sinus and extracranial subgaleal and periosteal vessels [Figure 2]a. Sinus pericranii was completely excised and communication with intracranial vessels was truncated at the dura mater. Bone defect was covered using split thickness autologous bone grafting [Figure 2]b. Surgery went uneventful with minimal blood loss and he was discharged in stable condition with no residual lesion [Figure 2]c or new onset sensorimotor deficits. Biopsy confirmed presence of vascular endothelium in specimen sent. At 3-month follow-up visit, he had surgical outcome with acceptable cosmesis and no recurrence or fresh neurologic deficits.
First described as "varix spurious circumscriptus venae diploicae frontalis" by Hecker in 1845, [4] and later as "Sinus Pericranii" by Stromeyer in 1850, [5] is an anomalous trans-diploic communication between intra-cranial and trans-cranial vessels through dilated emissary veins. It is classified as congenital, spontaneous or traumatic. [6] Recent evidence suggests that the spontaneous type is a delayed presentation of congenital variety, which can be differentiated from traumatic variant by histo-pathological evidence of differential lining of lesion wall. [7],[8] Its association with other intracranial intracranial vascular malformations particularly developmental venous anomaly, von Hippel-Lindau syndrome and blue nevus syndrome supports the malformation theory. [9],[10] Raised intracranial pressure especially chronic venous hypertension has been considered as one of the possible risk factor for congenital variant and this hypothesis is supported by its association with craniosynostosis and spontaneous regression of sinus pericranii after bony correction to alleviate intracranial hypertension. [10],[11],[12],[13],[14] Mechanical theory with traumatic avulsion of emissary veins is the most accepted hypothesis for traumatic cases. [4],[5],[15] Various classification patterns based on the size of venous communication, nature of circulation and compressibility status have been described. [16],[17],[18]
Sinus pericranii, affects predominantly males aged <30 years and is almost always located in midline frontal region draining into superior sagittal sinus. Off midline, posterior and lateral location is exceedingly rare. [3],[10],[11] Differentials include growing skull fracture, arterio-venous malformation, meningocele, encephalocele and intra-diploic dermoid. [19] Angiographically sinus pericranii is described as either dominant or accessory depending upon its drainage pattern and is an important factor in planning management. [10] Traditionally surgical intervention in the form of sac excision, trans-cranial venous anastomotic channel blockage and reinforcement/replacement of the underlying bone or minimally invasive endovascular neuro-intervention has been advocated for usually asymptomatic patients with cosmetic disfigurement. [13],[14],[19],[20]
More recently, there has been a paradigm shift in the management of sinus pericranii patients conservatively, considering sinus pericranii as normal and often necessary venous shunting whose disconnection from the brain could have grave consequence. [10],[11],[12] Angiographic evidence of dependence of venous outflow of brain on dominant sinus pericranii or developmental venous anomaly draining into accessory sinus pericranii is important contraindication for surgical removal. [12] "Squeezed out sinus syndrome" associated with craniosynostosis, achondroplasia with jugular foramen stenosis, brain tumor with sinus invasion, idiopathic intracranial hypertension with sinus thrombosis, Chiari malformation More Details, and idiopathic sinus obstruction is also relative contraindication owing to risk of massive intra-operative hemorrhage. [14] Park et al. [14] in their study have reported three risk factors for hemorrhage in patients with sinus pericranii: (1) Multiplicity or size (>6 cm) of sinus pericranii; (2) multiplicity (including sieve like forms) or size (>3 mm) of the trans-cranial channel; and (3) drainer-type sinus pericranii. Other potential life threatening complication include air embolism from opening of dilated venous channels. Spontaneous resolution of sinus pericranii has been reported after alleviating primary cause of raised intracranial pressure. [14],[21]
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[Figure 1], [Figure 2]
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